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Subcutaneous panniculitis-like T-cell lymphoma

ORPHA86884

Synonym(s)

SPTCLSubcutaneous panniculitic T-cell lymphoma

Prevalence

Unknown

Inheritance

Not applicable

Age of onset

All ages

ICD-10

C83.6

OMIM

-

UMLS

-

MeSH

C537503

MedDRA

-

Summary

Disease definition

Subcutaneous panniculitis-like T-cell lymphoma (SPTCL) is a rare cytotoxic cutaneous lymphoma that has been recognized as a distinct subset of peripheral T-cell lymphomas originating and presenting primarily in the subcutaneous fat tissue.

Epidemiology

The exact prevalence is unknown. A slight female preponderance has been reported

Clinical description

SPTCL is characterized by solitary or multiple erythematous subcutaneous nodules and plaques (proliferation of T lymphocytes and macrophages in the subcutaneous fat lobule), which appear in crops and are localized to the lower extremities or may be more generalized. Systemic manifestations include fever, chills, malaise, weight loss, hepatosplenomegaly, mucosal ulcers, and serosal effusions. SPTCL may be accompanied by hemophagocytic syndrome (characterized by the proliferation of histiocytes and phagocytosis of blood elements, hepatosplenomegaly, and coagulopathy and reported in approximately 30-40% of cases ; see these term), which may be fatal.

Etiology

The etiology is not completely understood. A relationship between subcutaneous T-cell lymphoma and cytophagic histiocytic panniculitis has been raised and still under discussion.

Diagnostic methods

Repeated biopsies may be required to establish the diagnosis.

Differential diagnosis

The differential diagnosis includes other lymphomas as well as atypical lobular panniculitis and cytophagic histiocytic panniculitis (see these term).

Management and treatment

SPTCL may be rapidly fatal or indolent. Most patients respond to systemic chemotherapy (cyclophosphamide, doxorubicin, vincristine and prednisone) or local radiation therapy.Last update: March 2007

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